Behçet’s Disease

Question 1

Define Behçet’s disease

Answer 1

A vasculitis of large, medium and small blood vessels, affecting arteries, veins and capillaries. It is often characterised by a triad of oral ulcers, genital ulcers and uveitis

Question 2

What are the causes/risk factors of Behçet’s disease?

Answer 2

• Aetiology is not known. Family studies show a possible genetic predisposition –HLA B51.
• Some studies suggest molecular mimicry after antibodies that develop against H. Pylori bind to antigens on blood vessels, resulting in autoimmune inflammation.

Risk Factors:
• Young: 20-40 years.
• Family history of Behçet’s disease.
• Mediterranean, Middle Eastern or Turkish origin.

Question 3

What are the signs and symptoms of Behçet’s disease?

Answer 3

• Oral ulcers - recurrent Apthous ulcers that are slow-healing and almost always present.
• Genital ulcers near the scrotum, labia or anus.
• Eye Manifestations:
- Anterior uveitis: Painful decrease in vision, red conjunctiva and a hypopyon –sterile pus due to infiltration to the anterior part of the eye.
- Posterior uveitis: Painless decrease in vision and floaters.
- Optic nerve involvement
• Erythema Nodosum
• Folliculitis
• Abdominal discomfort mimicking IBS and an incompetent ileocaecal valve.
• Joint and bone abnormalities.
• Meningitis
• Cardiac manifestations e.g. aortic regurgitation and pericarditis

Question 4

What investigations are carried out for Behçet’s disease??

Answer 4

• Pathergy Test - a subcutaneous skin prick is performed using a 21-gauge sterile needle, usually in the forearm, and observed for the formation of a papule or pustule in 48 hours. Pathergy test is positive in up to 60% of patients, with highest frequency in those of Middle Eastern origin.
• AI Screen - to exclude other AI diseases.
• CT/MRI/Endoscopy - to visualise lesions/ pathologies in the brain/ lungs and GI tract